Hypoplastic Left Heart Syndrome (HLHS) is a rare congenital heart defect in which the left side of the heart is severely under-developed. The structures of the heart that are usually affected include the mitral valve, left ventricle, aortic valve and the aorta. Because the left side of the heart is unable to send enough blood to the body, the right side of the heart must maintain the circulation for both the lungs and the body. This extra work eventually causes the right side of the heart to fail. Approximately 20% of children with HLHS have other birth defects or genetic syndromes. Children with HLHS are at increased risk for developing endocarditis and multiple other complications following their surgical procedures.

Diagnostic testing: A physical exam showing signs of heart failure (faster than normal heart rate, lethargy, liver enlargement, and rapid breathing) weak pulses at various locations (wrist, groin, and others); and abnormal heart sounds when listening to the chest.

Testing may include:

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  Cardiac catheterization;

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  Electrocardiogram (ECG);

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  Echocardiogram; and

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  Other imaging studies as appropriate.

Physical findings: Symptoms of HLHS may include:

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  Bluish (cyanosis) or poor skin color;

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  Cold hands and feet (extremities);

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  Lethargy;

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  Poor pulse;

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  Poor suckling and feeding;

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  Pounding heart;

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  Rapid breathing; and

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  Shortness of breath.        

ICD-9: 746.7

This congenital disease is fatal if not surgically treated during the first weeks of life. A newborn with this defect appears normal at birth. Symptoms usually occur in the first few hours of life and include bluish or pale skin color (cyanosis), cold hands and feet, lethargy, and poor feeding. Surgical intervention is generally a series of three staged surgical procedures. The child may need a heart transplant as time goes on. Congenital heart defects can be chronic conditions with health implications across the lifespan. Children with HLHS who survive into adulthood may require additional surgical intervention. Lifelong medical follow-up by a cardiologist will be required.

Palliative surgical intervention is often in the first years of life in a series of three staged surgical procedures. The first surgical procedure called the Norwood operation is done within the first few days of life. A second surgical procedure called the Glenn shunt or hemi-Fontan procedure is often done when the child is 4 to 6 months of age. The final surgical stage, stage III, is called the Fontan procedure. It is performed when the child is 18 months to 3 years of age. Additional surgeries, including possible heart transplantation may be required in surviving children and adults.

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  Clinical history and examination that describes the diagnostic features of the impairment;

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  Operative reports;

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  Cardiology consultation report; and

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  Imaging studies.